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The Successful Anesthetic Management of a Cesarean Delivery in a Patient with Fanconi–Bickel Syndrome

INTRODUCTION: Fanconi–Bickel syndrome (FBS) is a rare genetic condition characterized by extremely short stature, renal tubular dysfunction, osteoporosis, and rickets. The literature is scant regarding the successful reproduction of women with FBS. Cesarean delivery is indicated due to the risk of p...

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Autores principales: DeLeon, Alexander M., Gaiha, Rishi D., Peralta, Feyce M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9286953/
https://www.ncbi.nlm.nih.gov/pubmed/35846393
http://dx.doi.org/10.1155/2022/3220486
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author DeLeon, Alexander M.
Gaiha, Rishi D.
Peralta, Feyce M.
author_facet DeLeon, Alexander M.
Gaiha, Rishi D.
Peralta, Feyce M.
author_sort DeLeon, Alexander M.
collection PubMed
description INTRODUCTION: Fanconi–Bickel syndrome (FBS) is a rare genetic condition characterized by extremely short stature, renal tubular dysfunction, osteoporosis, and rickets. The literature is scant regarding the successful reproduction of women with FBS. Cesarean delivery is indicated due to the risk of pelvic fracture from vaginal delivery in a patient with FBS and rickets, yet no case describing the anesthetic has been reported. Clinical Findings. We present a 39-inch-tall, 46.47 kg/m(2) BMI woman with FBS who was scheduled for cesarean delivery and requesting neuraxial anesthesia. A low-dose, combined spinal-epidural technique (CSE) was employed to account for her extreme short stature yet allow for additional dosing if needed. The cesarean delivery, hospital course, and follow-up were all uneventful. CONCLUSION: A single case of an FBS patient's successful pregnancy was previously reported in the literature without describing the anesthetic technique. Our case is unique in that it is the first case in the literature that we are aware of describing the anesthetic technique. This case may provide a template for clinicians treating FBS patients and patients with extremely short stature.
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spelling pubmed-92869532022-07-16 The Successful Anesthetic Management of a Cesarean Delivery in a Patient with Fanconi–Bickel Syndrome DeLeon, Alexander M. Gaiha, Rishi D. Peralta, Feyce M. Case Rep Anesthesiol Case Report INTRODUCTION: Fanconi–Bickel syndrome (FBS) is a rare genetic condition characterized by extremely short stature, renal tubular dysfunction, osteoporosis, and rickets. The literature is scant regarding the successful reproduction of women with FBS. Cesarean delivery is indicated due to the risk of pelvic fracture from vaginal delivery in a patient with FBS and rickets, yet no case describing the anesthetic has been reported. Clinical Findings. We present a 39-inch-tall, 46.47 kg/m(2) BMI woman with FBS who was scheduled for cesarean delivery and requesting neuraxial anesthesia. A low-dose, combined spinal-epidural technique (CSE) was employed to account for her extreme short stature yet allow for additional dosing if needed. The cesarean delivery, hospital course, and follow-up were all uneventful. CONCLUSION: A single case of an FBS patient's successful pregnancy was previously reported in the literature without describing the anesthetic technique. Our case is unique in that it is the first case in the literature that we are aware of describing the anesthetic technique. This case may provide a template for clinicians treating FBS patients and patients with extremely short stature. Hindawi 2022-07-08 /pmc/articles/PMC9286953/ /pubmed/35846393 http://dx.doi.org/10.1155/2022/3220486 Text en Copyright © 2022 Alexander M. DeLeon et al. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
DeLeon, Alexander M.
Gaiha, Rishi D.
Peralta, Feyce M.
The Successful Anesthetic Management of a Cesarean Delivery in a Patient with Fanconi–Bickel Syndrome
title The Successful Anesthetic Management of a Cesarean Delivery in a Patient with Fanconi–Bickel Syndrome
title_full The Successful Anesthetic Management of a Cesarean Delivery in a Patient with Fanconi–Bickel Syndrome
title_fullStr The Successful Anesthetic Management of a Cesarean Delivery in a Patient with Fanconi–Bickel Syndrome
title_full_unstemmed The Successful Anesthetic Management of a Cesarean Delivery in a Patient with Fanconi–Bickel Syndrome
title_short The Successful Anesthetic Management of a Cesarean Delivery in a Patient with Fanconi–Bickel Syndrome
title_sort successful anesthetic management of a cesarean delivery in a patient with fanconi–bickel syndrome
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9286953/
https://www.ncbi.nlm.nih.gov/pubmed/35846393
http://dx.doi.org/10.1155/2022/3220486
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